The Facts

Lupus, also called systemic lupus erythematosus (SLE), is a chronic inflammatory condition that can affect any part of the body, including the skin, joints, kidneys, heart, lungs, and nervous system. It is one of the most common autoimmune diseases (diseases in which the immune system attacks its own body tissues).

Lupus is an unpredictable lifelong condition that typically affects young women between 18 and 40 years of age, but it can affect men and those older or younger. The ratio of women to men with lupus aged 15 to 40 is 12:1. Lupus occurs in 1 in 10,000 men, 1 in 1,000 white women, and 1 in 250 women of African descent.

In the early days of treating lupus, doctors only recognized the most severe cases and there were very limited treatments. As a result, the survival rate was not very good. Today, doctors usually recognize cases much earlier, and more mild cases, and there are now better ways of managing the disease. As a result, while there is still no cure for lupus, the survival rate is close to 90% 10 years after diagnosis.

However, available treatments all have risks and side effects, so people with lupus sometimes have to choose between those risks and the effects of their disease. In some cases, the disease is more moderate and minimal treatment is needed.

Causes

It's generally believed that lupus is caused by alterations in the immune system. The body's immune system normally fights foreign bacteria and viruses; however, with lupus, the immune system may fail to recognize "self" substances and will make antibodies that attack the body's own tissue. This is called autoimmunity.

The exact cause of lupus is still unknown. Multiple factors are involved in the development of the disease, including heredity and environmental factors. It is recognized that sunlight causes symptoms to flare up. Other triggers include viral infections, the stress of illness, sometimes pregnancy, and certain medications. Because more women are affected than men, another theory suggests a relationship with estrogens.

Symptoms and Complications

With lupus, symptoms may flare up every once in a while and then go away for a period of time. This symptom-free period is called remission.

Lupus may be mild or severe, and may result in a range of symptoms such as:

joint pain, swelling, and redness that comes and goes (the fingers and wrists are commonly affected)

rashes, especially across the nose and cheeks - known as a "butterfly rash"

fever

weight loss

chest pains due to inflammation of the heart and lungs (serositis)

coughing and breathing problems

sensitivity to sunlight, which can sometimes occur even with the use of sun block

unexplained fatigue

Raynaud's phenomenon (fingers or toes turn blue on exposure to cold, then white, then red and warm as the blood flow returns to normal)

hair loss

headaches

disordered thinking or confusion

labile emotions (unsteady and uncontrolled emotions or mood changes)

Complications of lupus may involve inflammation that can affect other areas of the body (such as the kidneys, central nervous system, and heart). If complications occur, they usually appear during the first few years after the initial diagnosis.

Kidney inflammation as a result of lupus is usually without symptoms at first, and unfortunately many people may not even notice it until the problem is advanced. Once it progresses, there may be other signs such as bloating, ankle swelling, and abnormal blood and urine tests. Ultimately kidney failure may develop.

Your doctor should closely monitor you for signs of early kidney disease, such as protein and other abnormalities in the urine.

Lupus also commonly accelerates and worsens atherosclerosis (hardening of the arteries), so your doctor may also monitor for risk factors like high blood pressure, cholesterol, and diabetes. Your doctor may also advise you to not smoke. It is very important to get treatment for atherosclerosis.

Making the Diagnosis

An antinuclear antibody (ANA) test is the most important test for lupus, as almost all people with lupus will have elevated blood levels of antinuclear antibodies. However, a diagnosis will not be based on ANA results alone, because many people have positive ANA tests without lupus, and it can also occur in other autoimmune diseases. A person with a positive ANA test who does not have clinical signs or other lab abnormalities has about a 5% chance of developing full-blown lupus in their lifetime.

Your medical history and a physical examination done by your doctor will play an important role in making the diagnosis. Other laboratory studies such as tests of kidney function, as well as joint X-rays and MRI (magnetic resonance imaging) scans, will help determine the extent of the disease.

People may need heart tests and an electroencephalogram (a test to measure electrical activity of the brain) to detect neurolupus (lupus that affects the brain).

Treatment and Prevention

The use of medication to treat lupus depends on the severity of the disease. In some cases, medication may not even be necessary.

Commonly prescribed medications include:

painkillers such as nonsteroidal anti-inflammatory drugs (NSAIDs) - some of these medications are available over-the-counter, but check with your doctor or pharmacist before starting one

hydroxychloroquine* is used, although it is often in combination with other medications to help control lupus

oral corticosteroids are the main treatment for most cases - this type of medication will help to reduce inflammation and its symptoms

immunosuppressive agents (e.g., cyclophosphamide, mycophenolate, azathioprine) suppress the immune system from attacking the body's organs and tissues - these are used when the kidney, brain, or other major organs are involved

Although all of these medications can be helpful and sometimes even life-saving, they have potentially very serious side effects. You should discuss the benefits and risks carefully with your doctor. You may also want to discuss your medications with health care professionals who are experienced in their use, such as rheumatologists (doctors who specialize in the diagnosis and treatment of arthritis and other diseases that affect the joints, muscles, and bones).

For those who have lupus, the following tips may be helpful:

Consult a rheumatologist. Rheumatologists have the most experience and expertise in diagnosing and managing lupus in its many forms.

Pregnancy can sometimes trigger the onset of lupus or it may worsen it if you already have lupus, but in other cases the disease may not be affected by pregnancy. If you have lupus and are planning to become pregnant, or if you develop lupus while pregnant, you should be under the care of both an obstetrician or gynecologist experienced in high-risk pregnancy as well as a rheumatologist.

Get plenty of rest and relaxation.

Learn stress management methods.

Receive regular medical and dental care.

Participate in regular moderate exercise.

Maintain a healthy, balanced diet.

Do not smoke.

Maintain vitamin D levels well within the recommended guidelines.

Don't take over-the-counter medications without the advice of a health professional.

If you're taking corticosteroids and other immune-suppressing medications, report any signs of infection to your doctor.

Avoid excessive exposure to the sunlight's ultraviolet rays - wear hats, sunglasses, and long-sleeved clothing and use a sunscreen with a sun protection factor (SPF) of at least 15 that protects against both ultraviolet A and B (UVA and UVB) light.

Consider joining a support group to help cope with various aspects of the condition.

Inform yourself as much as possible about lupus.

*All medications have both common (generic) and brand names. The brand name is what a specific manufacturer calls the product (e.g., Tylenol®). The common name is the medical name for the medication (e.g., acetaminophen). A medication may have many brand names, but only one common name. This article lists medications by their common names. For information on a given medication, check our Drug Information database. For more information on brand names, speak with your doctor or pharmacist.